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A 15 year old girl with a history of asthma had, at the age of 9 years, a diagnosis of idiopathic dilated cardiomyopathy (IDCM). On admission the patient was asymptomatic, with no medical treatment. Peripheral blood eosinophilia was absent. Resting ECG revealed a sinus rhythm with a left bundle branch block. Two dimensional (cross sectional) echocardiography showed a globus and dilated left ventricle with mildly reduced left ventricular (LV) function (LV ejection fraction 48%). No echogenic apical mass or endocardial thickening of one or both ventricles were visible. Cardiac catheterisation showed a moderate increase of LV end diastolic pressure with a dip–plateau pattern. Unpredictably, LV angiography revealed a localised filling defect of the posterior wall with complete obliteration of the LV apex (right anterior oblique view, top = diastole, bottom = systole), without mitral valve regurgitation. Right heart catheterisation showed a mild increase of systolic and mean pulmonary pressures and, at angiography, the ventricle had normal dimensions and function. Biventricular endomyocardial biopsy was performed and, at histology, a discrepancy between left and right ventricular features was evident. Right ventricular histology was compatible with the diagnosis of IDCM. Conversely, histology of the left ventricle showed a notably thickened endocardium (1.2 mm), with a three zonal layering appearance (dense fibrous (A), loose fibrous (B), and granulating tissue layers (C)) suggestive of endomyocardial fibrosis. Surgical treatment was not considered, as the patient was asymptomatic and no mitral regurgitation was present. This case should alert physicians to be cautious when a child presents with a suspected IDCM and to take into consideration unusual disorders, such as endomyocardial fibrosis.